Complete stable remission and autoantibody specificity in myasthenia gravis

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Abstract

Objectives: Patients with myasthenia gravis (MG) are subgrouped as acetylcholine receptor (AChR)- positive, muscle-specific kinase (MuSK)-positive, and AChR/MuSK-negative MG (or double negative [DN]) on the basis of autoantibody assay. We investigated the relationships between autoantibody specificity, main clinical features, and outcome of the disease, in particular the occurrence of complete stable remission (CSR), by means of a retrospective study on a cohort of 677 Italian patients withMG. Methods: A total of 517 (76%) patients with AChR-positiveMG, 55 (8%) patients with MuSK-positive MG, and 105 (16%) patients with DN MG were included in the study. Kaplan-Meier and Cox proportional hazard regression analyses were used to evaluate associations between baseline characteristics, antibody specificity, and CSR. Results: Clinical stage at onset and at maximal worsening was more severe for MuSK-positive patients: bulbar impairment at maximal worsening was found in 83.6% of MuSK-positive patients compared with 58.6% of AChR-positive patients and 43.8% of DN patients (p <0.001). Baseline characteristics of AChR-positive and DN patients were similar. CSR was observed in 3.6% of MuSKpositive patients compared with 22.2% of AChR-positive and 21.9% of DN patients. In the whole MG cohort, onset before age 40 (hazard ratio [HR] = 1.96, 95% confidence interval [CI] 1.27-3.02, p= 0.002) and ocular and generalized clinical stages at maximalworsening were associatedwith CSR (ocular, HR = 8.05, 95% CI 1.88-34.53, p = 0.005; generalized, HR = 3.71, 95% CI 1.16-11.90, p = 0.023; bulbar, HR = 3.16, 95% CI 1.00-10.05, p = 0.051). Conclusions: MuSK antibodies identify a clinically distinguishable, more severe form of MG since the disease onset, with a lower occurrence of CSR. These features should be considered by the clinician in the management of this particular form of MG.

Original languageEnglish
Pages (from-to)188-195
Number of pages8
JournalNeurology
Volume80
Issue number2
DOIs
Publication statusPublished - Jan 8 2013

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Myasthenia Gravis
Autoantibodies
Cholinergic Receptors
Phosphotransferases
Muscles
Confidence Intervals
Remission
Specificity
Antibody Specificity
Age of Onset
Retrospective Studies
Regression Analysis
Double Negatives
Hazard

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

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Complete stable remission and autoantibody specificity in myasthenia gravis. / Baggi, Fulvio; Andreetta, Francesca; Maggi, Lorenzo; Confalonieri, Paolo; Morandi, Lucia; Salerno, Franco; Bernasconi, Pia; Montomoli, Cristina; Barberis, Massimo; Mantegazza, Renato; Antozzi, Carlo.

In: Neurology, Vol. 80, No. 2, 08.01.2013, p. 188-195.

Research output: Contribution to journalArticle

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title = "Complete stable remission and autoantibody specificity in myasthenia gravis",
abstract = "Objectives: Patients with myasthenia gravis (MG) are subgrouped as acetylcholine receptor (AChR)- positive, muscle-specific kinase (MuSK)-positive, and AChR/MuSK-negative MG (or double negative [DN]) on the basis of autoantibody assay. We investigated the relationships between autoantibody specificity, main clinical features, and outcome of the disease, in particular the occurrence of complete stable remission (CSR), by means of a retrospective study on a cohort of 677 Italian patients withMG. Methods: A total of 517 (76{\%}) patients with AChR-positiveMG, 55 (8{\%}) patients with MuSK-positive MG, and 105 (16{\%}) patients with DN MG were included in the study. Kaplan-Meier and Cox proportional hazard regression analyses were used to evaluate associations between baseline characteristics, antibody specificity, and CSR. Results: Clinical stage at onset and at maximal worsening was more severe for MuSK-positive patients: bulbar impairment at maximal worsening was found in 83.6{\%} of MuSK-positive patients compared with 58.6{\%} of AChR-positive patients and 43.8{\%} of DN patients (p <0.001). Baseline characteristics of AChR-positive and DN patients were similar. CSR was observed in 3.6{\%} of MuSKpositive patients compared with 22.2{\%} of AChR-positive and 21.9{\%} of DN patients. In the whole MG cohort, onset before age 40 (hazard ratio [HR] = 1.96, 95{\%} confidence interval [CI] 1.27-3.02, p= 0.002) and ocular and generalized clinical stages at maximalworsening were associatedwith CSR (ocular, HR = 8.05, 95{\%} CI 1.88-34.53, p = 0.005; generalized, HR = 3.71, 95{\%} CI 1.16-11.90, p = 0.023; bulbar, HR = 3.16, 95{\%} CI 1.00-10.05, p = 0.051). Conclusions: MuSK antibodies identify a clinically distinguishable, more severe form of MG since the disease onset, with a lower occurrence of CSR. These features should be considered by the clinician in the management of this particular form of MG.",
author = "Fulvio Baggi and Francesca Andreetta and Lorenzo Maggi and Paolo Confalonieri and Lucia Morandi and Franco Salerno and Pia Bernasconi and Cristina Montomoli and Massimo Barberis and Renato Mantegazza and Carlo Antozzi",
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T1 - Complete stable remission and autoantibody specificity in myasthenia gravis

AU - Baggi, Fulvio

AU - Andreetta, Francesca

AU - Maggi, Lorenzo

AU - Confalonieri, Paolo

AU - Morandi, Lucia

AU - Salerno, Franco

AU - Bernasconi, Pia

AU - Montomoli, Cristina

AU - Barberis, Massimo

AU - Mantegazza, Renato

AU - Antozzi, Carlo

PY - 2013/1/8

Y1 - 2013/1/8

N2 - Objectives: Patients with myasthenia gravis (MG) are subgrouped as acetylcholine receptor (AChR)- positive, muscle-specific kinase (MuSK)-positive, and AChR/MuSK-negative MG (or double negative [DN]) on the basis of autoantibody assay. We investigated the relationships between autoantibody specificity, main clinical features, and outcome of the disease, in particular the occurrence of complete stable remission (CSR), by means of a retrospective study on a cohort of 677 Italian patients withMG. Methods: A total of 517 (76%) patients with AChR-positiveMG, 55 (8%) patients with MuSK-positive MG, and 105 (16%) patients with DN MG were included in the study. Kaplan-Meier and Cox proportional hazard regression analyses were used to evaluate associations between baseline characteristics, antibody specificity, and CSR. Results: Clinical stage at onset and at maximal worsening was more severe for MuSK-positive patients: bulbar impairment at maximal worsening was found in 83.6% of MuSK-positive patients compared with 58.6% of AChR-positive patients and 43.8% of DN patients (p <0.001). Baseline characteristics of AChR-positive and DN patients were similar. CSR was observed in 3.6% of MuSKpositive patients compared with 22.2% of AChR-positive and 21.9% of DN patients. In the whole MG cohort, onset before age 40 (hazard ratio [HR] = 1.96, 95% confidence interval [CI] 1.27-3.02, p= 0.002) and ocular and generalized clinical stages at maximalworsening were associatedwith CSR (ocular, HR = 8.05, 95% CI 1.88-34.53, p = 0.005; generalized, HR = 3.71, 95% CI 1.16-11.90, p = 0.023; bulbar, HR = 3.16, 95% CI 1.00-10.05, p = 0.051). Conclusions: MuSK antibodies identify a clinically distinguishable, more severe form of MG since the disease onset, with a lower occurrence of CSR. These features should be considered by the clinician in the management of this particular form of MG.

AB - Objectives: Patients with myasthenia gravis (MG) are subgrouped as acetylcholine receptor (AChR)- positive, muscle-specific kinase (MuSK)-positive, and AChR/MuSK-negative MG (or double negative [DN]) on the basis of autoantibody assay. We investigated the relationships between autoantibody specificity, main clinical features, and outcome of the disease, in particular the occurrence of complete stable remission (CSR), by means of a retrospective study on a cohort of 677 Italian patients withMG. Methods: A total of 517 (76%) patients with AChR-positiveMG, 55 (8%) patients with MuSK-positive MG, and 105 (16%) patients with DN MG were included in the study. Kaplan-Meier and Cox proportional hazard regression analyses were used to evaluate associations between baseline characteristics, antibody specificity, and CSR. Results: Clinical stage at onset and at maximal worsening was more severe for MuSK-positive patients: bulbar impairment at maximal worsening was found in 83.6% of MuSK-positive patients compared with 58.6% of AChR-positive patients and 43.8% of DN patients (p <0.001). Baseline characteristics of AChR-positive and DN patients were similar. CSR was observed in 3.6% of MuSKpositive patients compared with 22.2% of AChR-positive and 21.9% of DN patients. In the whole MG cohort, onset before age 40 (hazard ratio [HR] = 1.96, 95% confidence interval [CI] 1.27-3.02, p= 0.002) and ocular and generalized clinical stages at maximalworsening were associatedwith CSR (ocular, HR = 8.05, 95% CI 1.88-34.53, p = 0.005; generalized, HR = 3.71, 95% CI 1.16-11.90, p = 0.023; bulbar, HR = 3.16, 95% CI 1.00-10.05, p = 0.051). Conclusions: MuSK antibodies identify a clinically distinguishable, more severe form of MG since the disease onset, with a lower occurrence of CSR. These features should be considered by the clinician in the management of this particular form of MG.

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